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Alternating Hemiplegia of Childhood (AHC) » Clinical Presentation and Diagnosis
Age of Onset and Symptoms
AHC is a complex clinical condition with predominantly neurological manifestations.

The characteristic symptoms of the disease occur from early infancy. The most frequent first symptoms, that can be noted within the first days after birth, are episodes of abnormal eye movements (AEM). Dystonia and hemiplegia as well as other episodic neurological symptoms, appear in the following months.Sweney at all, 2009 proposed the following criteria for suspected AHC in early infancy:
  • focal or unilateral paroxysmal dystonia and/or flaccid hemiplegia in the first 6 months of life
  • paroxysmal ocular movements including biocular and monocular nystagmus and/or deviation in the first 3 month of life
  • absence of epileptiform changes during ictal events
In the course of the disease other neurological symptoms appear episodes of quadriplegia, dysphagia , autonomic disturbances , abnormal limb movements, epileptic seizures
  • interictal neurologic abnormalities such as tone abnormalities, choreoathetosis, and/or ataxia
  • persistent motor deficits such as spastic quadriplegia or hypotonia
  • developmental delay and intellectual disability
Electrocardiogram abnormalities are   recently reported to be common for AHC patients  and suggest that Cardiac dysfunction   may be another prominent feature of AHC disease. 
The type, the duration and the severity of the symptoms are specific for each individual with AHC.
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